Primary Hepatoid Carcinoma of the Pancreas: A Clinicopathological Study of 3 Cases With Review of Additional 31 Cases in the Literature

Abstract

Primary pancreatic hepatoid carcinoma (PHC) is very rare. Here, we reported 3 such cases with review of additional 31 cases in the literature. Our 3 patients were male (83, 72, and 54 years old, respectively). Serum α-fetoprotein (AFP) was elevated in 1 patient (case 3, 8338 ng/mL) and not measured in the other two. The PHC in patient 1 (pathological stage pT2N0M0) and patient 2 (pT3N0M0) showed pure hepatocellular carcinoma (HCC)-like morphology, whereas in case 3 it was a PHC with true glandular differentiation (pT4N0M0). The diagnosis of PHC was confirmed with positive immunohistochemical staining in the tumor cells for AFP (2/3), Hep Par 1 (3/3), glypican-3 (2/3), arginase-1 (2/3), and Sal-like protein 4 (1/3). CD10 and polyclonal carcinoembryonic antigen stains show focal canalicular pattern in 2/3 tumors. Patient 1 did not receive further treatment after resection and was alive with no evidence of disease at 107 months. Patient 2 died of postoperative complications, whereas patient 3 received postsurgical chemoradiation and died of disease at 29 months. Our findings and literature review indicate that PHCs can be divided into 4 histological subtypes: with pure HCC-like morphology (n = 22), with neuroendocrine differentiation (n = 8), with true glandular differentiation (n = 3), and with acinar cell differentiation (n = 1). On univariate analysis, pure HCC-like morphology was associated with better disease-specific survival (DSS; P = .04), whereas lymph node and distant metastases were associated with worse DSS ( P = .002 for both). Age, gender, presenting symptoms, serum AFP level, and T stage were not associated with DSS. On multivariate analysis, none of these parameters was significantly associated with DSS.